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06 February 2011, Volume 26 Issue 02 Previous Issue    Next Issue

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Follow-up study of the efficacy of different therapies on vasovagal syncope in children with different hemodynamic patterns. ZHANG Feng-wen，LIAO Ying，LI Xue-ying，CHEN Li，JIN Hong-fang，DU Jun-bao 2011, 26(02): 97.  Abstract ( )   To explore the individualized treatment for vasovagal syncope （VVS） in children with different hemodynamic patterns.Methods　A total of 63 children aged 6～19 years ［mean age （11.57±2.89） years］ with VVS were followed up in clinics or by telephone.Totally 32 cases were diagnosed as VVS vasodepressor type，31 cases were diagnosed as VVS mixed type or cardioinhibitory type. Fourteen cases took oral rehydration salts，13 cases took metoprolol and 36 cases took midodrine hydrochloride as initial treatment. Patients were followed up for 3～48 months，with a mean of （21±12） months. The recurrence of syncope in 3 months was studied to evaluate short-term efficacy of 3 different therapies. Taking recurrence of syncope as  ents，Kaplan-Meier curves were drawn to compare long-term efficacy of different therapies in treating VVS children. Results　There was no statistical difference for the short-term efficacy among the diffrent hemodynemic patterns when treated with oral rehydration salts，or metoprolol or midodrine hydrochloride （oral rehydration：χ2＝ 0.498，P > 0.05；metoprol：χ2＝ 0.627，P > 0.05；midodrine hydrochloride：χ2＝ 2.057，P > 0.05）. There was no statistical difference for the long-term efficacy among VVS children with different hemodynamic patterns when treated with oral rehydration salts，or metoprolol. Patients with mixed or cardioin hibitory patterns had a better response than those with vasodepressor pattern to midodrine hydrochloride（P < 0.01） Conclusions Oral rehydration salts，metoprolol or midodrine hydrochloride are appropriate to treat children with VVS. Children with VVS mixed and cardioinhibitory patterns are more appropriate to take midodrine hydrochloride as initial treatment.

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Postoperative follow-up of patients with complete atrioventricular septal defect complicated with pulmonary hypertension.　　 XIAO Ting-ting， LI Fen， SHEN Jie，HUANG Mei-rong. 2011, 26(02): 101.  Abstract ( )   To observe the operative efficacy in patients with complete atrioventricular septal defect compliated with pulmonary hypertension. Methods　From January 2003 to March 2007， CAVSD patients with pulmonary hypertension who underwent operative closure wereincluded in this study. Color Doppler with apical four-chamber view was used to evaluate the degree of pulmonary hypertension before surgery and 2 days， 1 month， 6 month and 1 year after the surgery. Cardiac catheterization was performed to evaluate pulmonary artery pressure and pulmonary arteriolar resistance （PAR） before surgery in patients whose age were over 6 months. The time of staying at ICU， ventilation time after surgery and the occurrence of pulmonary artery hypertension crisis were recorded. Results　A total of 75 CAVSD patients whounderwent operative closure were enrolled in this study. The mean staying time at ICU was （4.9±2.1）days， and the mean ventilation time was （2.1±0.9） days；12 patients developed pulmonary artery hypertension crisis after surgery. Patients with PAR > 8 wood unit were older，staying time at ICU and ventilation time were longer compared with patients with PAR < 8 wood unit （all P < 0.05）. The incidence of pulmonary artery hypertension crisis after surgery was also significantly higher in patients with PAR > 8 wood unit compared with patients with PAR < 8 wood unit（P < 0.05）. Five patients died. Four out of 5 hospital-dead patients had severe hypoplasia of the atrioventricular valve. Pulmonary artery pressure of 39 patients（52%） returned to normal two days after surgery， 51 patients（68%） returned to normal one month after surgery， 64 patients（85%） returned to normal 6 months after surgery， and 68 patients（91%） returned to normal one year after surgery； 7 patients have pulmonary hypertension one year after surgery. Conclusion　Our results suggest that early surgical repair for CAVC is safe and beneficial. Preoperative PAR > 8 wood unit is associated with increased risk of pulmonary artery hypertension crisis after surgery.

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Population pharmacokinetics of high-dose methotrexate in childhood lymphoid malignancies. LIU Ling*，HUA Ying，ZHAO Wei-hong，LU Xin-tian，MA Yue-hong，LU Wei-wei，DU Jun-bao，L 2011, 26(02): 104.  Abstract ( )   To establish the population pharmacokinetic model of high-dose methotrexate（HDMTX）5 g/m2 in children with lymphoidmalignancies， and evaluate characteristics of pharmacokinetic parameters about the elimination delay of HDMTX 5 g/m2 . Methods　Fifty-one children with lymphoid malignancies received 194 courses of HDMTX 5 g/m2. Measure MTX in plasma by FPIA. An extended least squares approach to nonlinear mixed effects modeling（NONMEM）was used to estimate pharmacokinetic parameters，intersubject and residual variability. Explain this variability in terms of patient specific information such as age or body weight and so on. Evaluate characteristics of pharmacokinetic parameters about the elimination delay of HDMTX 5g/m2. Results　The following population parameters were obtained using a two-compartment open model. Clearance of central compartment（CL1）= 5.61 ×（1 - 0.0183 ×（Age-8.3）+ 0.00683 ×（WBC-7.12））L/（h·m2），inter- compartment clearance（CL2）= 0.239 L/（h·m2），central volume（V1）= 12.1 ×（1 + 0.0369 ×（BUN-3.52））L/m2，perilpheral volume（V2）=2.83 ×（1 + 0.00102 ×（PLT-254.6））L/m2. The constant of distribution phase was α，the average being 0.469/h，ranging from 0.14/h to 1.96/h.The constant of elimination phase was β ，the average being 1.645/h，ranging from 0.02/h to 0.3/h. The elimination phase started 7 distribution phases later. Both the inter-compartment clearance and the peripheralvolume of the elimination delay group were clearly larger than that of the non-elimination delay one. Conclusions　The WBC of patient may have influence on the clearance of central compartment，the BUN on the central volume and the PLT on the peripheral volume. There might be more MTX distributing to the peripheral volume，if elimination delay happened. As a result of rescue based on the measurement of MTX concentration in plasma，the toxicity effect of elimination delay group could be controlled.

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Association between antiepileptic drug-induced Stevens-Johnson syndrome and HLA-B*1502.　　 HE Xue-lian，WANG Fang-lin，KANG Shi-xiu，WU Ge-fei，SUN Dan，YAO Hui，HU Jia-sheng，MA 2011, 26(02): 108.  Abstract ( )   We mainly aimed to examine whether HLA-B*1502 gene is associated with Stevens-Johnson Syndrome （SJS） induced by antiepileptic drugs （AEDs） association in Han children in South China mainland. Methods　We enrolled 16 patients with cutaneous adverse drug reactions（cADRs）induced by antiepileptic drugs， including 5 patients with SJS，2 with hypersensitivity syndrome（HSS）and 9 with maculopapular eruption（MPE），from 2007 to 2009. We also included 32 AED-tolerant controls in 2：1 ratio with cases in age，sex，and AEDprescribed. We also included 38 healthy controls. Multiplex amplification refractory mutation system（ARMS-PCR）was used to determine theirgenotypes. Results　We found that 5 patients with SJS were HLA-B*1502 positive while only 2 individuals were positive in AED-tolerant and healthy controls，respectively. The allele frequency of HLA-B*1502 in patients with cADRs was significantly higher compared with AED-tolerant controls（33.3% vs 6.25%，P < 0.05，OR = 7.5，95% CI 1.25～44.89）or with general population（33.3% vs 7.89%，P < 0.05，OR = 5.30，95%CI 1.09 ～25.84）. If we confined patients with AED-induced SJS alone，stronger association between HLA-B*1502 and AED-induced SJS was found in patients （P < 0.0001，OR = 247.99，95%CI 5.87～3831.70）. When the AED-tolerant group was used as the control，HLA-B*1502 allele had 100% sensitivity （95% CI：81.5% ～100%） and 71.4% specificity （95% CI：30% ～95%） for testing AED-induced SJS. HLA-B*1502 was not associated with MPE and HSS. Conclusion　Our findings have further confirmed the strong association of HLA-B*1502 with AED-induced SJS/TEN in Chinese Han children in South China and HLA-B*1502 is a valuable marker for testing AED-induced SJS. This knowledge could be used to guide personalized medicine for children with epilepsy by screening HLA-B*1502 before prescribing AEDs.

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Effects of TanshinoneIIA on inflammatory cytokines and GMP-140 in the patients with Kawasaki disease.　　 ZHANG Qing，LI Xiao-jing，YU Jie，XU Ming，ZHOU Min，SHI Hong，HUANG Cheng，WANG Xue-me 2011, 26(02): 112.  Abstract ( )   To study the levels of serum IL-6，IL-8，IL-1β，TNF-α，GMP-140and the number of platelet in different treatment for patients with Kawasaki disease， and to investigate the mechanism of TanshinoneIIA in Kawasaki disease. Methods　Sixty-one patients with Kawasaki disease and nineteen healthy children as controls were recruited into the study. The patients with Kawasaki disease were randomly divided into two groups：TanIIA treatment group and contrast treatment group.The serum IL-6，IL-8，IL-1β，TNF-α and GMP-140 levels were detected by ELISE and peripheral blood platelet number was measured using automatic hematocyte analyzer. Results　The levels of serum IL-6， IL-8， IL-1β， TNF-α and GMP-140 were increased before treatment，and decreased after treatment of five to seven days.The difference was statistically significant.The number of platelet was slightly increased before treatment，and significantly increased after treatment of five to seven days. After treatment of five to seven days，the levels of serum IL-6， IL-1βand GMP-140 in the group of TanIIA treatment decreased significantly compared with those in the group of contrast treatment，and the difference was statistically significant. Concluson　TanIIA might diminish the damage of immune vasculitis and platelet activation by decreasing the levels of serum IL-6， IL-1βand GMP-140.

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Genetic consultancy and prenatal genetic diagnosis in a family of chronic renal failure caused by WT1 mutation. ZHANG Hong-wen， ZHANG Yan-qin， WANG Fang， YANG Hui-xia， DING Jie. 2011, 26(02): 115.  Abstract ( )   To explore the methods of prenatal genetic diagnosis in chronic renal failure caused by WT1 mutation. Methods　Prenatal genetic diagnosis was performed on the proband’s mother after genetic consultancy. Mutation analysis of the fetus was performed on NA-based level of amniocytes. Fetus sex was determined by PCR amplification of SRY as well as karyotypes analysis. Maternal cell contamination was excluded by linkage analysis with three polymorphic microsatellite markers on X chromosome. Results　There was a WT1 IVS9+5G > A （heterozygotic） and a NPHS2 860A > G （heterozygotic） in the proband， respectively. The mother didn’t carry the WT1 IVS9+5G > A but carried the NPHS2 860A > G mutation. The father carried neither of the mutation. Amniocytes genomic DNA level based prenatal diagnosis showed that the fetus did not carry the same mutation as the mother or the proband. PCR amplification of SRY and karyotpyes analysis showed a female fetus. Linkage analysis of X chromosome polymorphic microsatellite markers showed that there was no maternal cell contamination in amniocytes. Conclusion　We have established the technique of prenatal genetics diagnosis in chronic renal failure caused by WT1 mutation in China.

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The different lente glucocorticoid effect of remission induction on children with nephrotic syndrome.　　 ZHANG Bi-li*，LI Chen，LIN Shu-xiang，WANG Wen-hong，LI Li，ZHANG Xuan，FAN Shu-ying，D 2011, 26(02): 118.  Abstract ( )   To investigate the effects of different lente glucocorticoid on the primary nephrotic syndrome （PNS）children’s remission induction. Methods　54 PNS kid patients at the time of their initial treatment in Tianjin Children’s Hospital were randomly divided into three groups:prednisone group，triamcinolone group and methylprednisolone group； they were given corresponding GC treatment.Monitor 24-hour urinary protein（Upro）levels， Alb and Tcho. Record the Upro negative conversion time. Test the mRNA expression levels of the glucocorticoid receptor （GR）subtypes in PBMCs by semi-quantitative RT-PCR. Results　 ethylprednisolone was better than triamcinolone and prednisone on the 24 h Upro decrease and the increase of plasma Alb. Methylprednisolone and triamcinolone had a shorter Upro negative conversion time than prednisone group. The GRαup-regulation degree of methylprednisolone was better than triamcinolone and prednisone. Methylprednisolone and triamcinolone’s down-regulation ability of GRβ was superior to prednisone. Conclusion　After the GC treatment，the Upro negative conversion time，changes of Upro and Alb and the level of GR can be used as valuable indicators for efficacy assessment at remission induction period. The effects of methylprednisolone or triamcinolone are better than prednisone at the remission induction period of PNS in initial treatment.

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One case report and literature review of autoimmune lymphoproliferative syndrome.　　 HUANG Li-bin，ZHANG Li-dan，KE Zhi-yong，XU Ling-ling，LIU Yin，WANG Zhuo，LUO Xue-qun 2011, 26(02): 121.  Abstract ( )   To report the clinical features，its treatment and follow-up of one autoimmune lymphoproliferative syndrome （ALPS），and enhance the recognition of this rare disease in China. Methods　The clinical features，treatment and follow-up data of a case diagnosed as ALPS in our hospital were analyzed，and related literature was reviewed. Results　This 1-year-and-11- month old boy presented with pancytopenia，hepatosplenomegaly，and lymphadenopathy，as well as the clinical features of glomerulonephritis and inflammatory bowel disease.The percentage of CD3+CD4CD8 double negative T （DNT） cell increased markedly and had positive autoantibodies，such as PAIgG，ANA， ANCA and insulin autoantibody.The genes of Fas，Fas ligand and Casp10 were sequenced but no mutation was found.Glucocorticosteroid in combination with other immunosuppressive agents was effective in short term，but relapses occurred when glucocorticosteroid was tapered. Conclusion　The diagnosis of autoimmune lymphoproliferative syndrome in this case is clinically proper. It would reduce misdiagnosis rate if the recognition of this disease is enhanced.

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Kasabach-Merritt syndrome： report of ten cases.　　 XIA Hong-ping，HE Zhen-juan， ZHU Jian-xing，ZHU Yue-niu，ZHU Xiao-dong，XIE Wei，OUYA 2011, 26(02): 125.  Abstract ( )   To improve comprehension of the clinical manifestation and treatment of Kasabach-Merritt syndrome （KMS）. Methods　Ten patients with KMS who were admitted to Xinhua Hospital between January 2006 and June 2010 were studied. Their clinical characteristics， laboratory test，therapy as well as response to treatment were analyzed respectively.The related literature was also reviewed. Results　The etiology of KMS remained unknown.The histopathology of KMS was thrombocytopenia and disseminated intravascular coagulation （DIC），and was associated with massive haemangioma. The clinical presentation was diverse. The histological characteristic was frequently kaposiform haemangioendothelioma or tufted angiomas. Therapy included management of coagulopathy and thrombocytopenia，and elimination of the tumor， including surgery， embolism and medicine （steroids， α-interferon， immunosuppressant， etc）. Conclusion　KMS should be considered in patients presenting with a giant haemangioma and thrombocytopenia. Once the diagnosis is made， therapy should be given， including improvement of thrombocytopenia and DIC and different ways to eliminate haemangioma.

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relation between early manifestations and severity of chemotherapy-related infection in children with tumor.　　 ZHANG Xiao-li，KE Zhi-yong，HUANG Li-bin，LUO Xue-qun. 2011, 26(02): 128.  Abstract ( )   To discuss the relation between early manifestations and severity of chemotherapy-related infection in children with tumor. Methods　The manifestations of infection during or after chemotherapy in 346 cases of 136 children with tumor were analyzed，and the differences in early manifestations between severe and mild infection were compared. Results　One hundred and thirty-three cases had severe infection，and 213 cases had mild infection. The incidences of chill，ague，fatigue，poor peripheral circulation and neutropenia in severe infection were significantly higher than those in mild infection. The highest temperature and the level of CRP at 6 to 12 hours during fever in severe infection were significantly higher than those in mild infection ； on the other hand，the probability to be severe infection in children with intensive chemotherapy，chill，ague，fatigue，poor peripheral circulation，absolute neutrophil count （ANC）≤0.5×109/L，CRP ≥ 40 mg/L，temperature ≥ 39.5 ℃ was higher than that in those without these manifestations （P < 0.05）. Conclusions　Children with intensive chemotherapy，chill，ague，fatigue，poor peripheral circulation，absolute neutrophil count （ANC） ≤ 0.5×109/L，CRP ≥ 40 mg/L， temperature ≥ 39.5 ℃ are more frequently to have severe infection.

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Changes of oxygenation in children with obstructive sleep apnea hypopnea syndrome.　　 CHANG Li， CAO Ling. 2011, 26(02): 131.  Abstract ( )   To explore the changes of oxygenation in children with obstructive sleep apnea hypopnea syndrome（OSAHS）. Methods　A total of 141 children in Capital Institute of Pediatrics from Jul. 2009 to Mar. 2010 were included in this study. Apnea hypopnea index AHI） > 5 was the diagnostic criterion for children with OSAHS. The children were divided into OSAHS group and non-OSAHS group. We compared the oxygenation indexes between OSAHS group and non-OSAHS group and between NREM period and REM period in OSAHS group. Results　There were significant differences in nadir pulse oxygen saturation（SpO2） and oxygen desaturation index（ODI） between OSAHS group and non-OSAHS group （Z = -3.64 and-5.67，P < 0.01）. There was no significant difference in mean SpO2 between OSAHS group and non-OSAHS group（Z = -0.90，P > 0.05）. There were significant differences in ODI and desaturation time per hour between rapid eye movement（REM） period and non-rapid eye movement（NREM） period in OSAHS group（Z = -5.568，Z = -5.937，P < 0.05）. There were no significant differences in maximum oxygen desaturation and maximum oxygen desaturation time between REM period and NREM period in OSAHS group（t = 0.364，t = 1.868，t = -0.33，P > 0.05）. Conclusion　Oxygen desaturation happens in children with OSAHS， and frequency of oxygen desaturation is higher and time of oxygen desaturation is longerin in REM period thand in NREM period.